Zollinger-Ellison Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

ZES MCID: ZLL002 MIFTS: 53	Zollinger-Ellison Syndrome (ZES) Categories: Endocrine diseases, Gastrointestinal diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
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Aliases & Classifications for Zollinger-Ellison Syndrome

MalaCards integrated aliases for Zollinger-Ellison Syndrome:

Name: Zollinger-Ellison Syndrome ¹¹ ¹⁹ ⁵⁸ ⁷⁵ ⁵³ ⁴³ ¹⁴ ¹⁶ ⁷¹ ³³

Gastrinoma ¹⁹ ⁵⁸ ⁷¹

Pancreatic Ulcerogenic Tumor Syndrome ¹⁹

Ze - [zollinger-Ellison] Syndrome ³³

Z E Syndrome ¹⁹

Zes ¹⁹

Characteristics:

Prevelance:

1-9/1000000 (Europe, Denmark, Worldwide) 1-9/100000 (Europe) <1/1000000 (Ireland) ⁵⁸

Age Of Onset:

Adult,Elderly ⁵⁸

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Endocrine diseases Gastrointestinal diseases

See all MalaCards categories (disease lists)

ICD10: ³¹ ³²

Endocrine, nutritional and metabolic diseases

Other disorders of glucose regulation and pancreatic internal secretion

Other disorders of pancreatic internal secretion

Abnormal secretion of gastrin

ICD11: ³³

Endocrine, nutritional or metabolic diseases

Endocrine diseases

Other disorders of glucose regulation or pancreatic internal secretion

Abnormal secretion of gastrin

Zollinger-Ellison syndrome

Orphanet: ⁵⁸

Rare gastroenterological diseases

Rare endocrine diseases

External Ids:

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Summaries for Zollinger-Ellison Syndrome

GARD: ¹⁹ Zollinger-Ellison syndrome (ZES) is a condition in which tumors called gastrinomas in the pancreas and duodenum (part of the small intestine) cause high levels of the hormone gastrin in the blood. High levels of gastrin then cause production of too much stomach acid. Signs and symptoms may include abdominal pain, peptic ulcers, vomiting blood, and diarrhea. The tumors are sometimes cancerous and may spread to other areas of the body. In most cases, the cause of ZES is unknown. However, about 25-30% of gastrinomas are caused by an inherited condition called multiple endocrine neoplasia type 1 (MEN1).

MalaCards based summary: Zollinger-Ellison Syndrome, also known as gastrinoma, is related to gastrinoma and multiple endocrine neoplasia, and has symptoms including dyspepsia An important gene associated with Zollinger-Ellison Syndrome is GAST (Gastrin), and among its related pathways/superpathways are Class A/1 (Rhodopsin-like receptors) and GPCR downstream signalling. The drugs Dexlansoprazole and Lansoprazole have been mentioned in the context of this disorder. Affiliated tissues include pancreas, small intestine and pituitary, and related phenotypes are zollinger-ellison syndrome and peptic ulcer

Disease Ontology: ¹¹ A syndrome that is characterized by the development of a one or more tumors (gastrinoma) in the pancreas, duodenum, or both that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach.

Orphanet: ⁵⁸ Zollinger-Ellison syndrome (ZES) is characterized by severe peptic disease (ulcers/esophageal disease) caused by hypergastrinemia secondary to a gastrinoma resulting in increased gastric acid secretion.

Wikipedia: ⁷⁵ Zollinger-Ellison syndrome (Z-E syndrome) is a disease in which tumors cause the stomach to produce too... more...

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Related Diseases for Zollinger-Ellison Syndrome

Diseases related to Zollinger-Ellison Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 403)

#	Related Disease	Score	Top Affiliating Genes
1	gastrinoma	32.0	SST SCT MEN1 GRP GAST CHGA
2	multiple endocrine neoplasia	31.6	SST SCT MEN1 GAST CHGA
3	islet cell tumor	31.5	SST SCT MEN1 GHRH GAST CHGA
4	carcinoid tumors, intestinal	31.5	SST MEN1 GAST CHGA
5	hyperparathyroidism	31.4	SCT MEN1 GAST CHGA
6	esophagitis	31.2	NTS MLN HRH2 GAST
7	primary hyperparathyroidism	31.1	SCT MEN1 GAST CHGA
8	pancreatic endocrine carcinoma	31.1	SST MEN1 GAST CHGA
9	parathyroid adenoma	31.1	MEN1 GAST CHGA
10	gastric neuroendocrine neoplasm	30.9	SST MEN1 GAST CHGA ATP4A ATP12A
11	diarrhea	30.9	SST SCT NTS MLN HRH2 GAST
12	carcinoid syndrome	30.9	SST NTS GHRH CHGA
13	adenoma	30.9	SST MEN1 GHRH CHGA
14	insulinoma	30.9	SST SCT MEN1 GAST CHGA
15	duodenal ulcer	30.7	SST SCT MLN HRH2 GRP GAST
16	pituitary tumors	30.7	SST MEN1
17	multiple endocrine neoplasia, type i	30.7	SST SCT MEN1 GHRH GAST CHGA
18	conn's syndrome	30.7	SST MEN1 GHRH CHGA
19	acromegaly	30.5	SST MEN1 GHRH
20	hyperinsulinemic hypoglycemia	30.5	SST SCT MEN1 GAST CHGA
21	neuroendocrine tumor	30.5	SST SCT NTS MEN1 GRP GAST
22	gastric ulcer	30.5	SST HRH2 GAST EGF ATP12A
23	pituitary adenoma	30.5	SST MEN1 GHRH CHGA
24	glucagonoma	30.5	SST SCT GAST CHGA
25	hypoglycemia	30.4	SST SCT GHRH GAST CHGA
26	peptic ulcer disease	30.4	SST SCT MEN1 HRH2 GAST ATP4A
27	barrett esophagus	30.4	GAST ATP4A ATP12A
28	gastritis	30.4	SST SCT HRH2 GAST CHGA ATP4A
29	prolactinoma	30.3	SST MEN1 EGF CHGA
30	pyloric stenosis	30.3	SST MLN GAST
31	ectopic cushing syndrome	30.3	SST CHGA
32	secretory diarrhea	30.3	SST GAST
33	pituitary gland disease	30.3	SST MEN1 GHRH
34	irritable bowel syndrome	30.3	SST MLN CHGA
35	hyperinsulinemic hypoglycemia, familial, 2	30.2	SST SCT GAST CHGA
36	gastrojejunal ulcer	30.2	ATP4A ATP12A
37	pernicious anemia	30.2	SST GAST CHGA ATP4A ATP12A
38	small cell cancer of the lung	30.2	SST GRP GAST CHGA
39	neuroendocrine carcinoma	30.2	SST MEN1 GRP GAST CHGA
40	peptic esophagitis	30.1	MLN HRH2 GAST ATP4A ATP12A
41	somatostatinoma	30.1	SST MEN1 GHRH GAST CHGA
42	functioning pituitary adenoma	30.1	SST MEN1 GHRH
43	short bowel syndrome	30.0	SST NTS GAST EGF
44	celiac disease 1	30.0	SCT GAST ATP4A ATP12A
45	duodenum cancer	30.0	SST SCT MEN1 GAST CHGA
46	dumping syndrome	29.9	SST SCT NTS MLN GAST
47	gastrointestinal stromal tumor	29.8	SST MEN1 EGF CHGA
48	duodenitis	29.8	SST GAST ATP4A ATP12A
49	vitamin b12 deficiency	29.8	GAST ATP4A ATP12A
50	pancreatic cancer	29.8	SST SCT NTS MEN1 GRP GHRH

Graphical network of the top 20 diseases related to Zollinger-Ellison Syndrome:

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Symptoms & Phenotypes for Zollinger-Ellison Syndrome

Human phenotypes related to Zollinger-Ellison Syndrome:

⁵⁸ ³⁰ (show all 36)

#	Description	HPO Frequency	Orphanet Frequency	HPO Source Accession
1	zollinger-ellison syndrome ⁵⁸ ³⁰	Obligate (100%)	Obligate (100%)	HP:0002044
2	peptic ulcer ⁵⁸ ³⁰	Hallmark (90%)	Very frequent (99-80%)	HP:0004398
3	esophagitis ⁵⁸ ³⁰	Hallmark (90%)	Very frequent (99-80%)	HP:0100633
4	duodenal ulcer ⁵⁸ ³⁰	Hallmark (90%)	Very frequent (99-80%)	HP:0002588
5	nausea ⁵⁸ ³⁰	Hallmark (90%)	Very frequent (99-80%)	HP:0002018
6	episodic abdominal pain ⁵⁸ ³⁰	Hallmark (90%)	Very frequent (99-80%)	HP:0002574
7	weight loss ⁵⁸ ³⁰	Frequent (33%)	Frequent (79-30%)	HP:0001824
8	jaundice ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0000952
9	multiple lipomas ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0001012
10	hyperparathyroidism ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0000843
11	hypercalcemia ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0003072
12	erythema ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0010783
13	hematochezia ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0002573
14	intestinal obstruction ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0005214
15	lipoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0012032
16	pituitary growth hormone cell adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0011760
17	pituitary prolactin cell adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0006767
18	adrenocortical adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0008256
19	extrahepatic cholestasis ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0012334
20	increased urinary cortisol level ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0012030
21	pituitary corticotropic cell adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0008291
22	thyroid adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0000854
23	elevated circulating parathyroid hormone level ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0003165
24	pituitary null cell adenoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0011761
25	parathyroid hyperplasia ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0008208
26	glucagonoma ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0030404
27	increased glucagon level ⁵⁸ ³⁰	Occasional (7.5%)	Occasional (29-5%)	HP:0030688
28	increased circulating cortisol level ³⁰	Occasional (7.5%)		HP:0003118
29	elevated circulating growth hormone concentration ³⁰	Occasional (7.5%)		HP:0000845
30	adrenocortical carcinoma ⁵⁸ ³⁰	Very rare (1%)	Very rare (<4-1%)	HP:0006744
31	gastrointestinal hemorrhage ⁵⁸		Occasional (29-5%)
32	growth hormone excess ⁵⁸		Occasional (29-5%)
33	diarrhea ⁵⁸		Very frequent (99-80%)
34	hypercortisolism ⁵⁸		Occasional (29-5%)
35	neuroendocrine neoplasm ⁵⁸		Obligate (100%)
36	pituitary adenoma ⁵⁸		Occasional (29-5%)

UMLS symptoms related to Zollinger-Ellison Syndrome:

dyspepsia

GenomeRNAi Phenotypes related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Decreased viability	GR00249-S	9.4	HRH2 MLN
2	Decreased viability	GR00381-A-1	9.4	GRP HRH2 MLN
3	Decreased viability	GR00381-A-3	9.4	GRP HRH2 MLN
4	Decreased viability	GR00386-A-1	9.4	GRP MLN
5	Decreased viability	GR00402-S-2	9.4	GRP MLN

MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	digestive/alimentary	MP:0005381	9.17	ATP12A ATP4A EGF GAST HRH2 MEN1

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Drugs & Therapeutics for Zollinger-Ellison Syndrome

Drugs for Zollinger-Ellison Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 66)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Dexlansoprazole

Approved, Investigational

Phase 4

138530-94-6

9578005

Lansoprazole

Approved, Investigational

Phase 4

103577-45-3

3883

Anti-Ulcer Agents

Phase 4

Antacids

Phase 4

Proton Pump Inhibitors

Phase 4

Gastrointestinal Agents

Phase 4

Esomeprazole

Approved, Investigational, Vet_approved

Phase 3

73590-58-6, 119141-88-7

9568614 4594

Fluorouracil

Approved

Phase 2

51-21-8

3385

Pancrelipase

Approved, Investigational

Phase 2

53608-75-6

8519

Streptozocin

Approved, Investigational

Phase 2

18883-66-4, 66395-18-4

29327

Doxorubicin

Approved, Investigational

Phase 2

23214-92-8

31703

Octreotide

Approved, Investigational

Phase 2

83150-76-9

383414 6400441

Pantoprazole

Approved

Phase 2

102625-70-7, 138786-67-1

4679

Gefitinib

Approved, Investigational

Phase 2

184475-35-2

123631

Sorafenib

Approved, Investigational

Phase 2

284461-73-0

216239

Bevacizumab

Approved, Investigational

Phase 2

216974-75-3

135329020

Miconazole

Approved, Investigational, Vet_approved

Phase 2

22916-47-8

4189

Clotrimazole

Approved, Vet_approved

Phase 2

23593-75-1

2812

Capecitabine

Approved, Investigational

Phase 2

154361-50-9

60953

Temozolomide

Approved, Investigational

Phase 2

85622-93-1

5394

Somatostatin

Approved, Investigational

Phase 2

38916-34-6, 51110-01-1

53481605 16129706

Everolimus

Approved

Phase 2

159351-69-6

70789204 6442177

Sirolimus

Approved, Investigational

Phase 2

53123-88-9

5284616 6436030

Romidepsin

Approved, Investigational

Phase 2

128517-07-7

5352062

Pancreatin

Phase 2

Liposomal doxorubicin

Phase 2

Antiviral Agents

Phase 2

Anti-Infective Agents

Phase 2

interferons

Phase 2

Protein Kinase Inhibitors

Phase 2

Angiogenesis Inhibitors

Phase 2

Antineoplastic Agents, Immunological

Phase 2

Immunoglobulins, Intravenous

Phase 2

Immunoglobulins

Phase 2

Immunoglobulin G

Phase 2

Antifungal Agents

Phase 2

Antibodies, Monoclonal

Phase 2

Antibodies

Phase 2

Endothelial Growth Factors

Phase 2

Mitogens

Phase 2

Antineoplastic Agents, Hormonal

Phase 2

Antimetabolites

Phase 2

Alkylating Agents

Phase 2

Antineoplastic Agents, Alkylating

Phase 2

Radiopharmaceuticals

Phase 2

Edotreotide lutetium LU-177

Phase 2

Edotreotide

Phase 2

23724894

Anti-Bacterial Agents

Phase 2

Antibiotics, Antitubercular

Phase 2

Immunosuppressive Agents

Phase 2

Interventional clinical trials:

(show all 30)

#	Name	Status	NCT ID	Phase	Drugs
1	Long-Term Study of the Efficacy and Safety of Lansoprazole in the Treatment of Zollinger-Ellison and Other Acid Hypersecretors	Completed	NCT00204373	Phase 4	Lansoprazole (Prevacid)
2	A Phase III, Multicenter, Open-Label Study To Evaluate the Control of Gastric Acid Secretion With Esomeprazole In Patients With Gastric Acid Hypersecretory States Including Idiopathic Hypersecretion and Zollinger-Ellison Syndrome For 12 Months	Completed	NCT00079833	Phase 3	Esomeprazole magnesium (Nexium)
3	An Open-label, Parallel-group, Multi-centre, Phase I/III Study to Assess the Safety, Pharmacokinetics, Pharmacodynamics and Efficacy of Repeated Once-daily Oral Administration of D961H 10 mg and D961H 20 mg in Japanese Paediatric Patients 1 to 14 Years Old With Gastrointestinal Acid Related Diseases	Completed	NCT02153398	Phase 3	D961H sachet 10 mg;D961H capsule 10mg;D961H capsule 20 mg
4	Evaluation of Chemotherapy With Streptozotocin Combined With 5-Fluorouracil and Adriamycin in Patients With Zollinger-Ellison Syndrome and Metastatic Non-Beta-Islet Cell Neoplasm	Completed	NCT00001165	Phase 2	combined chemotherapy with streptozotocin, 5-fluorouracil, and doxorubicin
5	Evaluation of Treatment With Interferon, Octreotide, or Their Combination in Patients With Zollinger-Ellison Syndrome and Progressive Metastatic Non-B Islet Cell Neoplasm	Completed	NCT00001228	Phase 2	Interferon
6	The Use of Oral Omeprazole and Intravenous Pantoprazole in Patients With Hypersecretion of Gastric Acid	Completed	NCT00001191	Phase 2	Pantoprazole;Omeprazole
7	Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors	Completed	NCT02259725	Phase 2	regorafenib
8	A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors	Completed	NCT00075439	Phase 2	gefitinib
9	A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors	Completed	NCT00131911	Phase 2	sorafenib tosylate
10	A Phase 2 Trial of Temsirolimus and Bevacizumab in Patients With Endometrial, Ovarian, Hepatocellular Carcinoma, Carcinoid or Islet Cell Cancer	Completed	NCT01010126	Phase 2	Temsirolimus
11	Randomized Phase II Study of Everolimus Alone Versus Everolimus Plus Bevacizumab in Patients With Locally Advanced or Metastatic Pancreatic Neuroendocrine Tumors	Completed	NCT01229943	Phase 2	Everolimus;Octreotide Acetate
12	A Phase 2 Study of Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors	Completed	NCT01525082	Phase 2	Capecitabine;Temozolomide
13	A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma	Completed	NCT00454363	Phase 2	Pazopanib Hydrochloride
14	A Randomized Phase II Study of Temozolomide or Temozolomide and Capecitabine in Patients With Advanced Pancreatic Neuroendocrine Tumors	Active, not recruiting	NCT01824875	Phase 2	temozolomide;capecitabine
15	A Prospective Randomized Study of the Efficacy and Safety of 177Lu-DOTATOC With Either Standard or Personalized Dosing for the Treatment of Patients With Somatostatin Receptor Positive NETs	Not yet recruiting	NCT04915144	Phase 2	177Lu-DOTATOC
16	A Pilot Trial of YF476, a Gastrin Antagonist, in Patients With Type II Gastric Carcinoids Associated With Zollinger-Ellison Syndrome	Terminated	NCT02454075	Phase 2	YF476
17	A Randomized, Double-Blinded, Placebo-Controlled Phase II Study of Adjuvant Everolimus Following the Resection of Metastatic Pancreatic Neuroendocrine Tumors to the Liver	Terminated	NCT02031536	Phase 2	everolimus
18	Phase II Evaluation of Real-Time, Pharmacokinetically Guided Everolimus in Patients With Hormone Receptor Positive Breast Cancer, Pancreatic Neuroendocrine Tumors (PNET), and Renal Cell Carcinoma	Terminated	NCT02273752	Phase 2	Everolimus
19	Phase II Study of Depsipeptide in Metastatic Neuroendocrine Tumors	Terminated	NCT00084461	Phase 2	romidepsin
20	Phase II Study of Dovitinib (TKI-258) in Progressive, Well-Differentiated Pancreatic Neuroendocrine Tumors With and Without Prior VEGF-Inhibitor Therapy	Withdrawn	NCT02108782	Phase 2	dovitinib lactate
21	A Phase I Trial of the mTOR Inhibitor RAD001 in Combination With VEGF Receptor Tyrosine Kinase Inhibitor PTK787/ZK 222584 in Patients With Advanced Solid Tumors	Completed	NCT00655655	Phase 1	everolimus;vatalanib
22	Phase I Feasibility Trial To Study The Safety Of Sealing Resected Pancreatic Surfaces After Partial Distal Pancreatectomy Using Laser Tissue Welding	Completed	NCT03147768	Phase 1
23	A Phase I Trial of Herceptin and Interleukin-12	Completed	NCT00004074	Phase 1
24	Phase I Study of Anti-IGF-1R Monoclonal Antibody, IMC-A12, and mTOR Inhibitor, Everolimus, in Advanced Low to Intermediate Grade Neuroendocrine Carcinoma	Completed	NCT01204476	Phase 1	Everolimus;Octreotide Acetate
25	A Phase 1 Study of Veliparib (ABT-888) in Combination With Capecitabine and Temozolomide in Advanced Well-Differentiated Neuroendocrine Tumors	Withdrawn	NCT02831179	Phase 1	Capecitabine;Temozolomide;Veliparib
26	Medical Therapy of Zollinger-Ellison Syndrome	Completed	NCT00001241
27	Evaluation of the Gastrinoma in Patients With Zollinger-Ellison Syndrome	Completed	NCT00001254
28	Diagnostic Evaluation of Patients With Suspected Abnormalities of Gastric Secretion	Completed	NCT00001240
29	Nexium Capsules Clinical Experience Investigation	Completed	NCT01434485
30	Evaluation of the Safety and Sensitivity of 68Ga-DOTATOC PET/CT for Imaging NET Patients	Recruiting	NCT03583528

Search NIH Clinical Center for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS ⁷¹ / NDF-RT ⁵⁰ :

Cimetidine

Cimetidine Hydrochloride

Famotidine

lansoprazole

Nizatidine

Omeprazole

Omeprazole 2 MG/ML Oral Suspension

Omeprazole magnesium

pantoprazole

Pantoprazole sodium

rabeprazole

Cochrane evidence based reviews: zollinger-ellison syndrome

Sources

Genetic Tests for Zollinger-Ellison Syndrome

Sources

Anatomical Context for Zollinger-Ellison Syndrome

Organs/tissues related to Zollinger-Ellison Syndrome:

MalaCards : Pancreas, Small Intestine, Pituitary, Liver, Thyroid, Lymph Node, Pancreatic Islet

Sources

Publications for Zollinger-Ellison Syndrome

Articles related to Zollinger-Ellison Syndrome:

(show top 50) (show all 2996)

#	Title	Authors	PMID	Year
1	[Zollinger-Ellison syndrome: a case report.]. ⁵³ ⁶²	Torres-Mansur R...Zamudio-Tiburcio H	20423792	2010
2	Diagnostic efficacy of the secretin stimulation test for the Zollinger-Ellison syndrome: an intra-individual comparison using different dosages in patients and controls. ⁵³ ⁶²	Kuiper P...Lamers CB	20299818	2010
3	False-positive secretin stimulation test for gastrinoma associated with the use of proton pump inhibitor therapy. ⁵³ ⁶²	Goldman JA...Wolfe MM	19245850	2009
4	Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report. ⁵³ ⁶²	Granberg D...Lehtihet M	18376130	2008
5	Secretin-receptor and secretin-receptor-variant expression in gastrinomas: correlation with clinical and tumoral features and secretin and calcium provocative test results. ⁵³ ⁶²	Long SH...Jensen RT	17711922	2007
6	Chromogranin A: is it a useful marker of neuroendocrine tumors? ⁵³ ⁶²	Campana D...Tomassetti P	17513802	2007
7	Diagnosis and differential diagnosis of hypergastrinemia. ⁵³ ⁶²	Arnold R	17985088	2007
8	Gastrinoma--morphological aspects. ⁵³ ⁶²	Kloppel G...Anlauf M	17985091	2007
9	Review article: Pathogenesis and management of gastric carcinoid tumours. ⁵³ ⁶²	Burkitt MD...Pritchard DM	17059512	2006
10	Increased islet beta cell replication adjacent to intrapancreatic gastrinomas in humans. ⁵³ ⁶²	Meier JJ...Butler PC	17016695	2006
11	Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. ⁵³ ⁶²	Anlauf M...Kloppel G	17006979	2006
12	[Pseudo Zollinger-Ellison syndrome in a patient with duodenal stenosis caused by tuberculosis]. ⁵³ ⁶²	Rautou PE...Ruszniewski P	16505764	2005
13	Double doses of secretin contribute to diagnosis of Zollinger-Ellison syndrome in secretin and selective arterial secretion injection tests--a case report. ⁵³ ⁶²	Yamamoto C...Sakisaka S	16240211	2005
14	Precursor lesions in patients with multiple endocrine neoplasia type 1-associated duodenal gastrinomas. ⁵³ ⁶²	Anlauf M...Kloppel G	15887103	2005
15	Intravenous calcium injection test is a novel complementary procedure in differential diagnosis for gastrinoma. ⁵³ ⁶²	Wada M...Imamura M	12205549	2002
16	The serological gastric biopsy: a non-endoscopical diagnostic approach in management of the dyspeptic patient: significance for primary care based on a survey of the literature. ⁵³ ⁶²	Korstanje A...Lamers CB	12408500	2002
17	Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. ⁵³ ⁶²	Gibril F...Jensen RT	11701693	2001
18	A randomized controlled crossover study comparing synthetic porcine and human secretins with biologically derived porcine secretin to diagnose Zollinger-Ellison Syndrome. ⁵³ ⁶²	Metz DC...Fein S	11328261	2001
19	Helicobacter pylori effects on gastritis, gastrin and enterochromaffin-like cells in Zollinger-Ellison syndrome and non-Zollinger-Ellison syndrome acid hypersecretors treated long-term with lansoprazole. ⁵³ ⁶²	Hirschowitz BI...Haber MM	11136282	2001
20	Curative resection of microgastrinomas based on the intraoperative secretin test. ⁵³ ⁶²	Kato M...Konishi J	11038217	2000
21	Bleeding from alimentary tract in pseudo Zollinger-Ellison syndrome. ⁵³ ⁶²	Kaminska B...Banach P	11208377	2000
22	Ovarian mucinous cystadenocarcinoma as a cause of Zollinger-Ellison syndrome: report of a case and review of the literature. ⁵³ ⁶²	Hirasawa K...Kaneko E	10811352	2000
23	Histomorphological characteristics of gastric mucosa in patients with Zollinger-Ellison syndrome or autoimmune gastric atrophy: role of gastrin and atrophying gastritis. ⁵³ ⁶²	Lehy T...Mignon M	10738314	2000
24	Fundic gland polyps: a still elusive entity on the eve of the year 2000. ⁵³ ⁶²	Declich P...Prada A	10833897	2000
25	The effect of Zollinger-Ellison syndrome and neuropeptide-secreting tumors on the stomach. ⁵³ ⁶²	Pisegna JR	10980995	1999
26	Prospective study of the value of serum chromogranin A or serum gastrin levels in the assessment of the presence, extent, or growth of gastrinomas. ⁵³ ⁶²	Goebel SU...Jensen RT	10193936	1999
27	Pancreatic endocrine tumors: recent advances. ⁵³ ⁶²	Jensen RT	10436815	1999
28	[The coexistence of 2 different neuroendocrine tumors of the upper gastrointestinal tract and pancreas]. ⁵³ ⁶²	Reusch J...Mohr HH	9861888	1998
29	Relationship between fundic endocrine cells and gastric acid secretion in hypersecretory duodenal ulcer diseases. ⁵³ ⁶²	Annibale B...Delle Fave G	9726392	1998
30	Hypergastrinemia and enterochromaffin-like cell hyperplasia. ⁵³ ⁶²	Delle Fave G...Annibale B	10461360	1998
31	[Gastric neuroendocrine tumor]. ⁵³ ⁶²	Diaz-Sanchez CL...Halabe-Cherem J	10068736	1998
32	Management of patients and subjects at risk for multiple endocrine neoplasia type 1: MEN 1. GENEM 1. Groupe d'Etude des Néoplasies Endocriniennes Multiples de type 1. ⁵³ ⁶²	Chanson P...Murat A	9167954	1997
33	Use of calcium provocative test in the diagnosis of gastroenteropancreatic endocrine tumors. ⁵³ ⁶²	Vezzadini C...Vezzadini P	9063035	1996
34	Adenocarcinoid of the liver arising within an area of hamartoma with predominant bile duct component. ⁵³ ⁶²	Papadogiannakis N...Seensalu R	8877646	1996
35	Prolonged hypergastrinemia does not increase the frequency of colonic neoplasia in patients with Zollinger-Ellison syndrome. ⁵³ ⁶²	Orbuch M...Jensen RT	8617144	1996
36	Serum gastrin and secretin test in Zollinger-Ellison syndrome. ⁵³ ⁶²	Moreira V...Erdozain JC	8551921	1996
37	Metastatic gastrinomas: localization with selective arterial injection of secretin. ⁵³ ⁶²	Gibril F...Jensen RT	8539410	1996
38	Endocrine tumors of the small and large intestine. ⁵³ ⁶²	Solcia E...Silini E	7479353	1995
39	Accuracy and precision of serum gastrin measurements in commercial laboratories. ⁵³ ⁶²	Notar-Francesco V...Straus EW	8520176	1995
40	Gastrinomas demonstrate amplification of the HER-2/neu proto-oncogene. ⁵³ ⁶²	Evers BM...Thompson JC	7911296	1994
41	Treatment of peptic ulcers from now to the millennium. ⁵³ ⁶²	Pounder RE	7949462	1994
42	Laboratory medicine in ulcer disease. ⁵³ ⁶²	Plebani M...Di Mario F	7923753	1994
43	[Somatostatin receptor scintigraphy in neuroendocrine tumors exemplified by a patient with hepatic metastases of gastrinoma]. ⁵³ ⁶²	Behr T...Wolf F	7513113	1994
44	The pathology of the gastrointestinal endocrine system. ⁵³ ⁶²	Solcia E...Capella C	8125073	1993
45	Parenteral control of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. ⁵³ ⁶²	Vinayek R...Jensen RT	8104773	1993
46	Management of patients with the Zollinger-Ellison syndrome. ⁵³ ⁶²	Mocek FW...Richardson JD	7901311	1993
47	Use of selective arterial secretin injection test to guide surgery in patients with Zollinger-Ellison syndrome. ⁵³ ⁶²	Imamura M...Takahashi K	8362526	1993
48	Ovarian cancers express and process progastrin. ⁵³ ⁶²	van Solinge WW...Rehfeld JF	8467501	1993
49	Chromogranin A and pancreastatin-like immunoreactivity in serum of gastrinoma patients. ⁵³ ⁶²	Syversen U...Waldum HL	8323758	1993
50	[Zollinger-Ellison syndrome with basal gastrin and non-diagnostic stimulation tests (secretin and calcium)]. ⁵³ ⁶²	Erdozain JC...Moreno A	8429704	1993

Sources

Genes for Zollinger-Ellison Syndrome

Genes related to Zollinger-Ellison Syndrome (0 elite genes):

(show all 14)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubMed IDs
1	GAST	Gastrin	Protein Coding	31.42	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	11136282 2103752 2188384 (more) 1940201 9726392 10980995 17985088 17985091 8125073 10833897 10068736 7911296 8467501 1358767 17006979 8877646 7513113 2101791 17016695 8323758 8429704 1697548 2184129 18376130 15887103 10738314 9322507 7479353 9861888 7923753 8520176 1888834 1694655 20423792 8617144 7901311 1475620 12408500 10811352 1514904 17059512 1925122 9063035 10461360 11208377
2	SCT	Secretin	Protein Coding	29.53	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	11328261 20299818 1992623 (more) 1624082 8539410 17711922 11038217 2230002 1396006 8520176 16240211 8429704 19245850 9167954 8551921 8362526 1888834 16505764 2294556 2166970 9063035 12205549
3	HRH2	Histamine Receptor H2	Protein Coding	26.29	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	7949462 8104773
4	SST	Somatostatin	Protein Coding	25.56	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	1672788 2188384 1358767
5	MEN1	Menin 1	Protein Coding	24.88	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Disorders (show sections)	11701693
6	CHGA	Chromogranin A	Protein Coding	24.83	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	17513802 2344924 8323758 (more) 10193936
7	GRP	Gastrin Releasing Peptide	Protein Coding	19.36	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	2101791
8	NTS	Neurotensin	Protein Coding	13.61	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
9	MLN	Motilin	Protein Coding	13.26	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
10	EGF	Epidermal Growth Factor	Protein Coding	11.93	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
11	GHRH	Growth Hormone Releasing Hormone	Protein Coding	9.76	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	10436815
12	ATP4A	ATPase H+/K+ Transporting Subunit Alpha	Protein Coding	8.51	DISEASES inferred ¹⁴
13	ATP12A	ATPase H+/K+ Transporting Non-Gastric Alpha2 Subunit	Protein Coding	8.5	DISEASES inferred ¹⁴
14	DNAH8	Dynein Axonemal Heavy Chain 8	Protein Coding	8.17	GeneCards inferred via : Publications (show sections)

Sources

Variations for Zollinger-Ellison Syndrome

Sources

Expression for Zollinger-Ellison Syndrome

Search GEO for disease gene expression data for Zollinger-Ellison Syndrome.

Sources

Pathways for Zollinger-Ellison Syndrome

Pathways related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	Class A/1 (Rhodopsin-like receptors) ⁶⁶ Show member pathways GPCR ligand binding ⁶⁶ GPCRs, class A rhodopsin-like ⁷⁴ Peptide GPCRs ⁷⁴ Peptide ligand-binding receptors ⁶⁶	12.83	SST SCT NTS MLN HRH2 GRP
2	GPCR downstream signalling ⁶⁶ Show member pathways G alpha (i) signalling events ⁶⁶ G alpha (q) signalling events ⁶⁶ Signaling by GPCR ⁶⁶	12.83	SST SCT NTS MLN HRH2 GRP
3	Signal Transduction ⁶⁶	12.74	SST SCT NTS MLN MEN1 HRH2
4	G alpha (s) signalling events ⁶⁶ Show member pathways Defective ACTH causes obesity and POMCD ⁶⁶	11.75	SCT HRH2 GHRH
5	Gastric acid production ⁷⁴	10.28	SCT GRP
6	Secretion of hydrochloric acid in parietal cells ⁷⁴	9.86	HRH2 ATP4A

Sources

GO Terms for Zollinger-Ellison Syndrome

Cellular components related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	extracellular space	GO:0005615	10.06	SST SCT GRP GHRH GAST EGF
2	extracellular region	GO:0005576	9.91	SST SCT NTS MLN GRP GHRH
3	neuronal dense core vesicle	GO:0098992	8.92	SST GRP CHGA

Biological processes related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	sodium ion export across plasma membrane	GO:0036376	9.56	ATP4A ATP12A
2	cellular potassium ion homeostasis	GO:0030007	9.46	ATP4A ATP12A
3	establishment or maintenance of transmembrane electrochemical gradient	GO:0010248	9.26	ATP4A ATP12A
4	positive regulation of phospholipase C-activating G protein-coupled receptor signaling pathway	GO:1900738	8.92	GRP CHGA

Molecular functions related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	P-type sodium:potassium-exchanging transporter activity	GO:0005391	9.62	ATP4A ATP12A
2	P-type potassium:proton transporter activity	GO:0008900	9.46	ATP4A ATP12A
3	neuropeptide hormone activity	GO:0005184	9.43	NTS GRP GHRH
4	hormone activity	GO:0005179	9.28	SST SCT MLN GHRH GAST
5	P-type potassium transmembrane transporter activity	GO:0008556	9.26	ATP4A ATP12A